a rare case of diffuse hemangiomatosis of the spleen with ...casereport a rare case of diffuse...

Case ReportA Rare Case of Diffuse Hemangiomatosis of the Spleen withSplenic Rupture following Aortic Valve Replacement

F. Capilli,1 M. Weinbeck,2 M. Czerny,2 M. Siepe,2 and T. Krauss1

1Department of Radiology, Section of Cardiovascular Radiology, University Hospital Freiburg, Freiburg, Germany2Department of Cardiovascular Surgery, Heart Center Freiburg, Bad Krozingen, Germany

Correspondence should be addressed to F. Capilli; [email protected]

Received 30 August 2016; Accepted 27 December 2016; Published 12 January 2017

Academic Editor: Suayib Yalcin

Copyright © 2017 F. Capilli et al.This is an open access article distributed under the Creative Commons Attribution License, whichpermits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

In this paper we present a rare case of splenic rupture that occurred after an open aortic valve replacement in a male patient withhemangiomatosis of the spleen and the liver. The patient was treated with an emergency splenectomy. He showed no other sings ofassociated systemic disorder, such as Klippel-trenaunay syndrome or Proteus syndrome.

1. Introduction

Splenic hemangiomas are the most common primary neo-plasm of the spleen. Usually they are asymptomatic inci-dental findings and can easily be diagnosed with computedtomography (CT) ormagnetic resonance imaging (MRI), dueto their typical contrast enhancement. As benign tumours,splenic hemangiomas usually do not require any treatment[1, 2]. Rarely, especially if localized under the splenic capsule,splenic hemangiomas can cause subcapsular hematomas,complete splenic rupture, and consequently severe internalbleeding. A diffuse splenic hemangiomatosis, which formsmultiple tumours within the whole spleen, is very rare andcan cause spontaneous splenic rupture [3].

2. Case Presentation

A 68-year-old man underwent an open aortic valve replace-ment because of severe aortic valve stenosis.The day after theopen-heart-operation a revision thoracotomy was needed,because of postoperative acute thoracic bleeding, and duringthe procedure a cardiopulmonary resuscitation was per-formed.

Three days after first surgery the patient became againhemodynamically unstable and presented signs of hemor-rhagic shock.

Sonography demonstrated an enlargement of the spleenand free fluid in the peritoneal space. Pericardial and pleuraleffusions could be excluded.

A multiphasic contrast-enhanced CT scan of the abdo-men confirmed a large amount of intraperitoneal bloodand an enlargement of the spleen caused by a subcapsularhematoma (Figure 1). The contrast-enhanced images of theCT scan (arterial and venous phase) demonstrated a het-erogenous contrast enhancement of the spleen and of the liverwith several hypervascular focal lesions in both organs, whichthen became more evident in the portal-venous phase of theCT scan (Figures 2 and 3). An acute perisplenic bleedingcould also be depicted, suggesting a splenic capsular tear(Figure 4). There was no evidence of rib fracture.

The patient underwent an emergency splenectomy withperioperative transfusion of 11 erythrocytes concentrates.During early postoperative care the patient was hemodynam-ically stable.

In the days following the abdominal procedure the patientsuffered from intermittent abdominal bleeding and under-went recurrent surgical treatment. 10 days after the initial pro-cedure the patient died due to the development of a multior-ganic failure.

The immunohistochemical analysis revealed the endo-thelial cells to be strong CD34-positive and also to expressfocally CD31. Factor8 was positive on the membranes. Many

HindawiCase Reports in RadiologyVolume 2017, Article ID 9164749, 3 pageshttps://doi.org/10.1155/2017/9164749

https://doi.org/10.1155/2017/9164749

2 Case Reports in Radiology

Figure 1: Nonenhanced CT scan of the abdomen shows blood in theperitoneal space (white arrows).

Figure 2: Arterial phase of contrast-enhanced CT scan depictsmultiple hypervascular lesions in the spleen (black arrow) and liver(white arrow).

macrophages were immunostained for CD68, while theendothelial cells remained mainly negative. CD8 was notexpressed in the most part of the vessels. The growth fractionwas circa 15%. The pathologist reported a “diffuse, mixedcavernous and capillary splenic hemangioma, affecting theentire spleen, with splenic capsular rupture.” Although nobiopsy of the liver was performed, the CT-appearance ishighly suggestive of hepatic involvement, leading to thediagnosis of a hemangiomatosis of the spleen and liver.

3. Discussion

The role of contrast-enhanced CT scan is paramount indepicting hypervascular splenic tumours, identifying typicalmalignant features, like aggressive growth with infiltration ofnear structures and area of necrosis of the angiosarcomas, anddiagnosing possible complication [4]. Because of the oftensimilar unspecific appearance of primary splenic lesions, suchas littoral cell angiomas, lymphangiomas, hamartomas, pelio-sis, hemangiopericytomas, and hemangioendotheliomas aswell as some hemangiomas themselves, a pathological andimmunohistochemical analysis is in many cases needed tosecure the diagnosis [5].

Splenic hemangiomatosis presents asmultifocal or diffuselocalization of splenic hemangiomas [6–9]. This condition isvery rare and is usually described in association with othersystemic disorders such as Klippel-trenaunay syndrome orProteus syndrome [10, 11]. Possible clinical presentations

Figure 3: Venous phase: multiple hepatic (white arrow) and splenic(black arrow) lesions.

Figure 4: Venous phase: extravasation of contrast agent suggestingperisplenic bleeding (white arrow).

include also Kasabach-Merritt syndrome, which consists ina consumptive coagulopathy due to large vascular tumours[12]. Spontaneous splenic ruptures are usually reported as acomplication of infectious diseases, hemopathies, or storagediseases associated with splenomegaly [13].

Delayed splenic injury is a very rare complication of CPR[14] and we see no clear temporal correlation between theCPR and the acute abdominal hemorrhage in our patient. Itcan be however supposed that extensive parenchymal dam-age, due to the hemangiomatosis, increased the vulnerabilityof the organ, exposing it to rupture even without a relevanttrauma. We suggest that the parenchymal disease of thespleen, the hemodynamic stress of an open-heart operation,and an anticoagulation therapy may have caused a delayedsplenic rupture in our patient after CPR.

4. Conclusion

This case illustrates a delayed splenic rupture in a patientwith hemangiomatosis of the spleen and the liver after openaortic valve replacement and CPR. Although very rare, thiscondition can possibly cause life-threatening complicationsafter CPR.

Competing Interests

The authors declare that they have no competing interests.

Case Reports in Radiology 3

Acknowledgments

The article processing charge was funded by the GermanResearch Foundation (DFG) andUniversity of Freiburg in thefunding programme Open Access Publishing.

References

[1] H. Steininger, D. Pfofe, L.Marquardt, H. Sauer, and R.Markwat,“Isolated diffuse hemangiomatosis of the spleen: case report andreview of literature,” Pathology Research and Practice, vol. 200,no. 6, pp. 479–485, 2004.

[2] M. R. Ambrosio, B. J. Rocca, N. Di Mari, A. Ambrosio, and S.Lazzi, “Multifocal capillary hemangioma (hemangiomatosis) ofthe spleen,” Tumori, vol. 98, no. 1, pp. e22–e26, 2012.

[3] F. Karakayali, C. Basaran, E. A. Soy et al., “Spontaneous spleenrupture and rectus sheath hematoma in a patient with Klippel-Trenaunay syndrome: report of a case,” Surgery Today, vol. 40,no. 2, pp. 154–157, 2010.

[4] R. M. Abbott, A. D. Levy, N. S. Aguilera, L. Gorospe, andW. M. Thompson, “Primary vascular neoplasms of the spleen:radiologic-pathologic correlation,” Radiographics, vol. 24, no. 4,pp. 1137–1163, 2004.

[5] C. I. Fotiadis, I. Georgopoulos, C. Stoidis, and P. Patapis, “Pri-mary tumors of the spleen,” International Journal of BiomedicalScience, vol. 5, no. 2, pp. 85–91, 2009.

[6] I. Makino, H. Tajima, H. Kitagawa, H. Nakagawara, and T.Ohta, “A rare case of hemangiomatosis of the spleen andintrapancreatic accessory spleen,” Abdominal Imaging, vol. 39,no. 6, pp. 1169–1174, 2014.

[7] C. N. Moss, J. A. Van Dyke, R. E. Koehler, and C. T. Smedberg,“Multiple cavernous hemangiomas of the spleen: CT findings,”Journal of Computer Assisted Tomography, vol. 10, no. 2, pp. 338–340, 1986.

[8] H. R. Latifi andM. J. Siegel, “Diffuse neonatal hemangiomatosis:CT findings in an adult,” Journal of Computer Assisted Tomog-raphy, vol. 16, no. 6, pp. 971–973, 1992.

[9] Y. Nakano, H. Fujisaki, T. Ishiguro et al., “Isolated diffusehemangiomatosis of the spleen with disseminated intravascu-lar coagulation: successful treatment with embolization andsplenectomy,” Journal of Pediatrics, vol. 166, no. 4, pp. 1093–1093.e1, 2015.

[10] S. Dekeyzer, B. Houthoofd, A. De Potter, M. Van Bockstal, P.Smeets, and D. Vogelaers, “Hemangiomatosis of the spleen ina patient with Klippel-trenaunay syndrome,” JBR-BTR, vol. 96,no. 6, pp. 357–359, 2013.

[11] Z.Wang, Z. Yu, Y. Su et al., “Kasabach-Merritt syndrome causedby giant hemangiomas of the spleen in patients with Proteussyndrome,” Blood Coagulation and Fibrinolysis, vol. 18, no. 5, pp.505–508, 2007.

[12] J.-Y. Tang, J. Chen, C. Pan, M.-Z. Yin, and M. Zhu, “Diffusecavernous hemangioma of the spleen with Kasabach-Merrittsyndrome misdiagnosed as idiopathic thrombocytopenia in achild,” World Journal of Pediatrics, vol. 4, no. 3, pp. 227–230,2008.

[13] P. Skok,M.Knehtl, D. Ceranic, and L.Glumbic, “Splenic rupturein systemic amyloidosis—case presentation and review of theliterature,” Zeitschrift fur Gastroenterologie, vol. 47, no. 3, pp.292–295, 2009.

[14] J. P. Pestaner and J. E. Smialek, “Splenic rupture followingcardiopulmonary resuscitation,” Resuscitation, vol. 54, no. 2, p.216, 2002.

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